scholarly journals Management of Pierre Robin sequence our experience and long-term follow-up

2017 ◽  
Vol 46 ◽  
pp. 252 ◽  
Author(s):  
S. Abuzinada ◽  
A. Alyamani
Keyword(s):  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Long Term Follow Up ◽  
Pierre Robin

scholarly journals Pierre Robin Sequence: Diagnostic Difficulties Faced while Differentiating Isolated and Syndromic Forms

2020 ◽  
Vol 63 (2) ◽  
pp. 86-90
Author(s):  
Girish Gulab Meshram ◽  
Neeraj Kaur ◽  
Kanwaljeet Singh Hura
Keyword(s):  
Positive Family History ◽  
Management Plan ◽  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Long Term Follow Up ◽  
Diagnostic Difficulties ◽  
History Of ◽  
Pierre Robin

Pierre Robin sequence (PRS) is characterized by the triad of retrognathia, glossoptosis, and airway obstruction. PRS may occur in isolation or in conjunction with other syndromes. Distinguishing isolated and syndromic forms of PRS helps clinicians decide the management plan. We describe two cases of PRS of Indian ethnicity and describe some of the difficulties that we faced while distinguishing isolated PRS from syndromic PRS. Both cases had a similar clinical presentation. However, one of the cases had a positive family history of congenital deafness and cleft palate, whereas the other case had apparent upper limb anomalies. These facts heightened the suspicion of an associated syndrome. However, based on the available facts and after thorough investigations, a tentative diagnosis of isolated PRS was made for both the patients. Both the cases were managed conservatively and were advised a long-term follow-up. When the associated anomalies are few, minor or concealed at birth, longitudinal follow-up of all PRS cases combined with thorough diagnostics including chromosomal analysis could help differentiate syndromic PRS from isolated PRS. Regardless, all cases of PRS require a multidisciplinary approach.

A Case Report of Absent Epiglottis in Children with Nager Syndrome: Its Impact on Swallowing

2017 ◽  
Vol 54 (6) ◽  
pp. 754-757 ◽  
Author(s):  
Sok Yan Tay ◽  
Woei Shyang Loh ◽  
Thiam Chye Lim
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Clinical Report ◽  
Pierre Robin Sequence ◽  
Literature Report ◽  
Robin Sequence ◽  
Aspiration Risk ◽  
Nager Syndrome ◽  
Pierre Robin

Objective This clinical report is the first literature report exploring the issues of an absent epiglottis in children diagnosed with Nager syndrome. Absent epiglottis has previously been described in children with Pierre Robin sequence and other syndromes. Conclusion The function of the epiglottis and its contribution to swallowing has always been debated. In this article and from our literature review, we felt that absent epiglottis possibly contributed to the aspiration risk and may partly explain the long-term feeding issues in these children despite corrective surgeries.

Long Term Speech and Feeding Outcomes in Patients With Pierre Robin Sequence

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Alexander Morzycki ◽  
Curtis Budden ◽  
Samuel Skulsky ◽  
Loredana Cuglietta ◽  
Regan Guilfoyle
Keyword(s):  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Pierre Robin

Long-Term Orthognathic Considerations in the Pierre Robin Sequence Patient

2020 ◽  
Vol 146 (5) ◽  
pp. 599e-606e ◽  
Author(s):  
Miles J. Pfaff ◽  
Fransia De Leon ◽  
Laura Le ◽  
Christos Haveles ◽  
Claire Liu ◽  
...  
Keyword(s):  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Pierre Robin

scholarly journals Pierre Robin Sequence: Incidence of Speech-Correcting Surgeries and Fistula Formation

2019 ◽  
Vol 57 (3) ◽  
pp. 344-351
Author(s):  
Charlotta Gustafsson ◽  
Pia Vuola ◽  
Junnu Leikola ◽  
Arja Heliövaara
Keyword(s):  
Surgical Technique ◽  
Cleft Palate ◽  
Airway Obstruction ◽  
Data Retrieval ◽  
Fistula Formation ◽  
Pierre Robin Sequence ◽  
Causal Factors ◽  
Robin Sequence ◽  
Pierre Robin

Objective: Children with Pierre Robin sequence (PRS) and cleft palate have a high rate of velopharyngeal insufficiency (VPI) following primary palatoplasty. Our purpose was to determine the long-term incidence of speech-correcting surgeries (SCSs) and fistula rates in PRS after primary palatoplasty and the influence of possible causal factors. Design: A retrospective single-center, observational chart review study. Participants: After exclusion, the study cohort comprised 78 nonsyndromic PRS children (48 females) born between 1990 and 2009 and treated at the Cleft Palate and Craniofacial Center of Helsinki University Hospital, Finland. Causal factors included gender, surgeon, age at primary palatoplasty, surgical technique, airway obstruction in infancy, and cleft severity. We analyzed the outcome at age 8 years and at data retrieval, with a median follow-up of 14 years (range: 8-27 years). Results: Thirty-four (43.6%) children received SCS by age 8 years, and of the 19 (24.4%) postoperative fistulas, 6 (7.7%) underwent closure. At data retrieval, 37 (47.4%) children had undergone SCS and 8 (10.3%) had a fistula closure. Median age at SCS was 6 years. The results showed no significant association for gender, surgeon, age at primary palatoplasty, surgical technique, cleft severity, or airway obstruction in infancy regarding incidence of SCS, fistulas, or repaired fistulas. Conclusion: Pierre Robin sequence in children is associated with a high incidence of SCS and fistula formation, which necessitates accurate clinical follow-up and observation of speech development. The development of VPI in PRS is complex and most likely involving multiple factors.

Airway Management in Pierre Robin Sequence

1998 ◽  
Vol 118 (5) ◽  
pp. 630-635 ◽  
Author(s):  
Charles M. Myer ◽  
J. Mark Reed ◽  
Robin T. Cotton ◽  
J. Paul Willging ◽  
Sally R. Shott
Keyword(s):  
Airway Obstruction ◽  
Scientific Data ◽  
Treatment Modalities ◽  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Methods Of Evaluation ◽  
Fellowship Programs ◽  
Pierre Robin ◽  
Term Management

There is a great deal of controversy regarding the long-term management of airway obstruction in children with Pierre Robin sequence. All 23 pediatric otolaryngology fellowship programs were surveyed to determine their current practice patterns. Issues that were addressed included methods of evaluation of airway obstruction, including polysomnography, choices for home care in children in whom observation and positioning treatment failed, and complications of the various treatment modalities. The lack of unanimity among the respondents mirrors the controversy found in the literature. Though tracheotomy appears to be favored as the method of long-term management, responses from those surveyed often were based on emotions as much as on scientific data. This report details those findings and offers suggestions to clinicians involved with the management of patients with Pierre Robin sequence with airway compromise. (Otolaryngol Head Neck Surg 1998;118:630–5.)

Gastroesophageal Reflux in Pierre Robin Sequence—Early Surgical Treatment

2000 ◽  
Vol 37 (2) ◽  
pp. 205-208 ◽  
Author(s):  
Zofia Dudkiewicz ◽  
Ewa Sekuła ◽  
Anna Nielepiec-Jałosińska
Keyword(s):  
Gastroesophageal Reflux ◽  
Pierre Robin Sequence ◽  
Upper Respiratory Tract ◽  
Floor Of The Mouth ◽  
Robin Sequence ◽  
Early Closure ◽  
Hospital Stays ◽  
Tract Infections ◽  
Pierre Robin

Objective To assess the prevalence of gastroesophageal reflux (GER) in patients with Pierre Robin sequence and to assess whether early palatoplasty with subperiosteal release of the floor of the mouth eliminates upper respiratory tract infections and gastroesophageal reflux. Methods In the period from 1993 to 1997, 24 children with Pierre Robin sequence were treated. Because GER is known to occur in patients with this syndrome, we screened for GER in each child for whom an early palatoplasty was to be performed at 3 months of age. In 21 cases, the presence of GER was confirmed. Within the past 2 years, palatoplasty has been combined with subperiosteal release of the floor of the mouth (SRFM). Prompt clinical improvement and shortened hospital stays have resulted. Results and Conclusions Follow-up studies performed several months after the surgery showed no evidence of GER. The authors conclude that an early closure of the impaired palatopharyngeal ring and the elimination of the mechanism that causes the posterior rotation of the tongue can reduce the need for such surgical procedures as glossopexy, gastrostomy, tracheostomy, and Nissen's fundoplication.

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